Cerebellar symptoms in motor neuron diseases. Special form of amyotrophic lateral sclerosis plus syndrome

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive and selective loss of motor neurons in the cerebral cortex, brainstem, and spinal cord. The upper motor neuron syndrome is characterized by symptoms of spastic paresis. Muscle weakness and atrophy, fasciculations, and cramps are typical signs for the degeneration of the lower motor neurons. In 1994, the El Escorial criteria were proposed for the diagnosis of ALS. These criteria include ALS-plus syndromes, which are defined by an association of ALS with extrapyramidal features or dementia. In this paper, we present two cases of ALS associated with signs of cerebellar degeneration. According to the revised El Escorial criteria, the described unusual combination of upper and lower motor neuron signs in association with cerebellar ataxia can be classified as a specific form of ALS-plus syndromes.     

Comparison of two procedures for meniscal cysts. A report of 35 patients with a mean follow-up of 33 months

BACKGROUND: The cause, pathogenesis, and appropriate treatment of meniscal cysts remain controversial. PURPOSE: We wanted to evaluate the results of treatment of meniscal cysts with two different operative procedures. STUDY DESIGN: Prospective cohort study. METHODS: Two different operative procedures were compared. Sixteen patients (group 1) had an open excision of the cyst performed in addition to arthroscopic examination of the knee. Nineteen patients (group 2) were treated entirely arthroscopically. The postoperative treatment course was the same for both groups. The mean length of follow-up was 33 months. RESULTS: The results were excellent or good in 86% of the patients (30 of 35) and were equally good for both procedures. Patients with degenerative changes of the knee joint seemed to have a less favorable outcome. CONCLUSIONS: Good or excellent results can be expected from either open or arthroscopic treatment of meniscal cysts.     

High pretreatment serum concentration of basic fibroblast growth factor is a predictor of poor prognosis in small cell lung cancer.

Basic fibroblast growth factor (bFGF) is a secreted multifunctional cytokine and a potent stimulator of angiogenesis. We measured bFGF concentrations from serum samples taken from 103 patients with small cell lung cancer at the time of diagnosis. Serum concentration of bFGF (S-bFGF) ranged from undetectable to 54 pg/ml (median, 6 pg/ml). S-bFGF was not associated with age, sex, performance status, or stage. A high pretreatment S-bFGF was associated with poor overall survival. The 1- and 2-year survival rates of the patients within the highest quartile of S-bFGF (>or=17 pg/ml) were only 26% and 11%, respectively, in contrast to the 49% and 20% 1- and 2-year survival rates of those patients with S-bFGF < 17 pg/ml (P = 0.013). The 1- and 2-year survival rates of the patients with extensive-stage disease were 33% and 10%, respectively (P = 0.0091). Interestingly, S-bFGF provided additional prognostic information to the stage because the 1- and 2-year survival rates of patients with extensive-stage disease and a high S-bFGF (>or=17 pg/ml) were as low as 16% and 5%, respectively (P = 0.0026). Similarly, in the multivariate model of survival analysis, patients with both extensive-stage disease and a high S-bFGF (>or=17 pg/ml) were found to have a particularly poor prognosis (relative risk of death, 2.1; 95% confidence interval, 1.2-3.6; P = 0.0057). We conclude that a high S-bFGF at diagnosis is associated with poor outcome in small cell lung cancer, possibly reflecting active angiogenesis and rapid tumor growth, and may complement prognostic information obtained by staging.     

Neue Entwicklung vaginaler Inkontinenzoperationen

Vaginal repair has long been the procedure of choice for gynecologists to treat female urinary incontinence. High failure and recurrence rates in objectively controlled studies have guided towards abdominal procedures such as colposuspension and traditional slings. Tension-free vaginal tape (TVT) has proven to be an excellent choice in female stress urinary incontinence without prolapse or other pathology, especially in patients at risk. Severe paravaginal defects and additional pathology leave a wide spectrum for the traditional colposuspension. Further long-term follow-up and comparative studies will have to show whether the good results with TVT can be stabilized and which complications with the alloplastic sling material might arise. With many questions unanswered, we are very cautious with implanting TVT in very young women.     

Current issues in gastroeneterologic rehabilitation

In Hungary gastroenteric diseases' rehabilitation, which is one of the youngest branch of medical rehabilitation, has grown out of traditional, sanatorial care from the 70s. Its main feature that it is based on a biopsychosocial attitude towards patients as a team-work. Though it comprises traditional, medical diagnosis and treatment, medicine and medical technology are not the primary resources of gastroenteric diseases rehabilitation: it is mainly built on a friendly and co-operating relations with the patients, which helps doctors to exert their mental and intellectual influence promoting their patients recovery. Therefore, gastroenteric diseases' rehabilitation can be sharply separated from active treatment and care. The article gives full details of the possible methods of gastroenteric diseases' rehabilitation, such as diagnosis, medicinal treatment, psychotherapy, dietotherapy, physiotherapy and regimen guidance. In addition, it is emphasized that under sanatorial circumstances the effects of rehabilitation, especially of regimen guidance multiplies. Furthermore it gives numerical data about the present situation of gastroenteric diseases' rehabilitation in Hungary. Considering the data gained from the special rehabilitation hospital located in Visegrád, the article touches upon the illnesses calling for this kind of rehabilitative treatment not passing over the fact of multimorbidity, especially important in rehabilitation.     

Breast-saving therapy and primary reconstruction with latissimus dorsi flap combined with radiotherapy

OBJECTIVE: The use of latissimus-dorsi-flap with postoperative radiotherapy is method of choice in primary reconstruction of breast cancer. The efforts of radiotherapy on flap healing, cosmetic results and formation of edema in the arm were studied in 30 patients. MATERIALS AND METHODS: 30 patients were followed in three to six months intervals clinically and sonographically (ATL-Ultramark 9, HDI). RESULTS: The most frequent symptom was a moderate edema. No healing problems or interference with cosmetic results were observed. Blood flow in the thoraco-dorsal vessels showed unchanged pre- and postoperatively. CONCLUSIONS: The complains might be the consequence of the combination of surgical dissection of the axilla, radiotherapy and possible additional factors such as trauma and overstress for example. Cosmetic result and healing seems to be impaired by 50 to 60 gy.     

Technique and survival after laparoscopically assisted right hemicolectomy

Background The role of laparoscopic colon resection in the management of colon cancer is still controversial. In this article, the surgical strategy and techniques are described, with further consideration of the oncologically relevant aspects.Methods Between March 1993 and July 2003, we performed laparoscopic right hemicolectomy in 56 patients with right colon carcinoma. Average age was 74.5 years (range, 17–92). We performed a standardized surgical procedure that included mobilization from the vascularized mesenteric bridges with a window technique, transection of the ileocolic lymphovascular pedicle, and lateral and proximal mobilization of the ileocecum, ascending colon, right flexure, and proximale transversum. After enlargement of one of the trocar incisions the exteriorized colon was resected and an extracorporeal anastomosis was performed in the standard manner.Results There were no conversions to open. The mean operating time was 119 ± 38 min, the mean length of resected colon was 27.8 ± 4.48 cm, and the average width of the clear margins was 6.8 ± 5.3 cm. One patient died. Lymph nodes were positive in 21 patients. The 5-year survival rate in the 48 patients who were operated on with curative intent was 75%. We have had two local recurrences. The overall 5-year mortality-free fraction was 63%. Cox multivariate analysis showed that the mortality-prognostic factors were tumor stage and length of resected colon, whereas Kaplan-Meier analysis showed that the mortality-prognostic factors were positive lymph nodes and tumor stage.Conclusions Our results show that laparoscopic right hemicolectomy for colon cancer can be performed safely. Complications and recurrence rates are comparable to those for left-sided laparoscopic and open procedures. Therefore, we recommend this procedure as the method of choice. Laparoscopically treated patients with stage II and stage III disease have almost the same cumulative rate of survival.     

Tuberous sclerosis and the kidney: from mesenchyme to epithelium, and beyond

The renal manifestations of tuberous sclerosis complex (TSC) are remarkably diverse, including polycystic kidney disease, oncocytomas, renal cell carcinomas, and both benign and malignant angiomyolipomas. All of these occur in children as well as adults with TSC. Benign angiomyolipomas, which can cause spontaneous life-threatening hemorrhage, are by far the most prevalent and the greatest source of morbidity. What is particularly unusual about TSC, setting it apart from virtually all other inherited forms of renal disease, is the abnormalities of both mesenchymal cells (angiomyolipomas) and epithelial cells (cysts, oncocytomas, and carcinomas). Recently, the TSC1/TSC2 protein complex was shown to inhibit the kinase mTOR (mammalian target of rapamycin). This places TSC1/TSC2 at center stage in signaling pathways that regulate cell growth. Furthermore, recent advances in TSC1/TSC2 signaling open the door for targeted therapy for TSC patients. Here, we will address the genetic, cellular and biochemical mechanisms that may contribute to the unusually broad spectrum of renal disease in cells with TSC1 or TSC2 mutations, and consider how the TSC signaling pathways may be linked to other renal diseases such as polycystic kidney disease and renal cell carcinoma.